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Genetics of Carney complex and related familial lentiginoses, and other multiple tumor syndromes.
Front Biosci
March 2000
Unit on Genetics and Endocrinology, Developmental Endocrinology Branch, National Institute of Child Health & Human Development (NICHD), National Institutes of Health (NIH), Building 10, Room 10N262, Bethesda, MD 20892-1862, USA.
Carney complex is a multiple endocrine neoplasia (MEN) syndrome that affects the adrenal cortex, the pituitary and thyroid glands, and the gonads. The complex is also associated with skin and mucosa pigmentation abnormalities and myxoid and other neoplasms of mesenchymal and neural crest origin. Thus, this syndrome also belongs to another group of genetic disorders, the lentiginoses (or lentigenoses), which include the Peutz-Jeghers, LEOPARD, arterial dissections and lentiginosis, and Laugier-Hunziker syndromes, Cowden disease and Ruvalcaba-Myhre-Smith (Bannayan-Zonana) syndrome and the centrofacial, benign patterned and segmental lentiginoses, all of which can be associated with a variety of developmental defects.
View Article and Find Full Text PDFCarney complex: the complex of myxomas, spotty pigmentation, endocrine overactivity, and schwannomas.
Semin Dermatol
June 1995
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN 55905, USA.
The complex of myxomas, spotty pigmentation, endocrine overactivity, and schwannomas (the Carney complex) is a multisystem tumorous disorder that is transmitted as a mendelian autosomal dominant trait. Approximately 150 affected patients are known worldwide. The myxomas, which tend to be multiple in the involved organ, affect the heart, skin and breast.
View Article and Find Full Text PDFMultiple lentigines, myxoid tumours and endocrine overactivity; four cases of Carney's complex.
Br J Dermatol
April 1992
Department of Dermatology, Royal Victoria Hospital, Belfast, N. Ireland.
Four patients with Carney's complex, one sporadic and three familial, are described. The sporadic case was a young male with centrofacial lentigines, who developed cyclical Cushing's syndrome secondary to bilateral pigmented nodular adrenocortical disease, two separate left atrial myxomas, and buccal mucosal myxomas. The three familial cases, who all had varying degrees of centrofacial/mucosal lentigines and cutaneous myxoid tumours, were a woman with myxoid mammary fibroadenomatosis and a left atrial myxoma, her daughter who developed a prolactin-secreting pituitary adenoma, and her son who had bilateral large-cell calcified Sertoli cell testicular tumours, and an axillary psammomatous melanotic schwannoma.
View Article and Find Full Text PDFInherited patterned lentiginosis in blacks.
Arch Dermatol
September 1989
Dermatology Service, National Naval Medical Center, Bethesda, Md.
We present 10 patients with autosomal dominant transmission of a pigmentary pattern characterized by facial, lip, extremity, buttock, and palmoplantar small, discrete hyperpigmented macules. None of the patients had lesions of the oral mucosa or internal organ system abnormalities. These patients' pigmentary pattern can resemble the ones seen in other lentiginosis syndromes.
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